tbuckley/cabot-search huggingface dataset:
Efficacy and safety of dupilumab in bullous pemphigoid: a retrospective multicentric study of 36 patients
Authors: Parna Moghadam, Emmanuelle Tancrede, Jean-David Bouaziz, Julien Kallout, Christophe Bedane, Edouard Begon, Isabelle Bourgault-Villada, Andreea Calugareanu, Olivier Dereure, Fatma Jendoubi, Anne Pham-Ledard, Saskia Ingen-Housz-Oro, Catherine Picard-Dahan, Manuelle Viguier, Thibault Mahevas, Marie Jachiet, Estelle Charvet, Charles Cassius, Marina Alexandre, Clémence Lepelletier
Journal: British Journal of Dermatology (2023)

Bullous pemphigoid (BP) is the most common autoimmune blistering disease in Europe and its treatment can be challenging. Several published cases have reported dupilumab efficiency in treatment-refractory patients. We conducted a retrospective multicentric study of 36 patients to evaluate the real-life efficiency of dupilumab in BP. Our results suggest that dupilumab, in association with high-potency topical steroids, could be rapidly effective in various clinical forms of BP and seems to be well tolerated in the elderly.
Rethinking ‘Caucasian’: a push toward more equitable language in dermatology
Authors: Jeanette R Comstock, Olushola L Akinshemoyin Vaughn, Idowu Olugbade, Beth A Drolet
Journal: British Journal of Dermatology (2023)

The word ‘Caucasian’ is a commonly used adjective intended to describe a person with lightly pigmented skin. The term is not only an inaccurate descriptor for the majority of white people, but also has racist origins based on Johann Blumenbach’s racial hierarchy, which placed Caucasians at the top. We aim to educate our specialty about the history of the word ‘Caucasian’ and to empower dermatologists to choose accurate descriptors that are not steeped in racist historical origins.
Multiscale Anisotropic Scaffold Integrating 3D Printing and Electrospinning Techniques as a Heart‐on‐a‐Chip Platform for Evaluating Drug‐Induced Cardiotoxicity
Authors: Sitian Liu, Zihan Wang, Xinyi Chen, Mingying Han, Jie Xu, Ting Li, Liu Yu, Maoyu Qin, Meng Long, Mingchuan Li, Hongwu Zhang, Yanbing Li, Ling Wang, Wenhua Huang, Yaobin Wu
Journal: Advanced Healthcare Materials (2023)

Cardiac safety assessments are significant in drug discovery, as drug-induced cardiotoxicity (DIC) is the primary cause of drug attrition. Despite heart-on-a-chip (HoC) technology becoming an increasingly popular tool for evaluating DIC, its development remains a challenge owing to the anisotropic cardiac structure of the native myocardium. Herein, an anisotropic multiscale cardiac scaffold is presented via a hybrid biofabrication method by combining 3D printing with electrospinning technology, where the 3D-printed micrometer-scale scaffold frames enable mimicking the interwoven myocardium anatomical structure and the branched-aligned electrospun nanofibers network is able to directionally guide cellular arrangements. The in vitro 3D bioengineered cardiac tissues are then fabricated by encapsulating three-layer multiscale scaffolds within a photocurable methacrylated gelatin hydrogel shell. It is demonstrated that such an anisotropic multiscale structure could contribute to enhancing cardiomyocyte maturation and synchronous beating behavior. More attractively, with the integration of 3D bioengineered cardiac tissues and a self-designed microfluidic perfusion system, a 3D anisotropic HoC platform is established for evaluating DIC and cardioprotective efficacy. Collectively, these results indicate that the HoC model developed by integrating the 3D bioengineered cardiac tissues could effectively recapitulate the clinical manifestations, thereby highlighting their efficacy as a valuable preclinical platform for testing drug efficacy and cardiotoxicity.
Pulsed Field Ablation: Is It Better Than Conventional Thermal Ablation for Treatment of Atrial Fibrillation?
Authors: Andrea M. Russo
Journal: Circulation (2023)

ver since Haissaguerre and colleagues demonstrated that ectopic beats in the pulmonary veins can initiate episodes of atrial fibrillation (AF) which can be treated with radiofrequency ablation, electrophysiologists have been searching for more effective, safer, and efficient ways to ablate this arrhythmia.Several sources of energy delivery have been investigated, including radiofrequency, cryoballoon, laser, ultrasound, and now more recently pulsed field ablation (PFA).What are the reasons for the search for alternative energy sources?The cornerstone of catheter ablation of AF remains complete isolation of pulmonary veins that can be performed with point-by-point or single-shot ablation technology.The Achilles' heel of ablation is the unfortunate high rate of pulmonary vein reconnection and limited durability of lesions.
A Tale of Two Parallel Rhythms
Authors: Anshul R. Gupta, Abhishek J. Deshmukh, Freddy Del-Carpio Munoz
Journal: Circulation (2023)

A 54-year-old woman with a history of frequent symptomatic arrhythmias and Wolff-Parkinson-White syndrome status post prior catheter ablation procedures presented for evaluation of persistent symptoms.Despite previous successful procedures for concealed accessory pathway, slow atrioventricular nodal pathway, and premature ventricular contraction ablations, she continued to be symptomatic, with recurrent shortness of breath with associated palpitations, dizziness, fatigue, and limitations in her functional capacity.She was referred to us for further management.She had no other relevant history, and physical examination was unremarkable apart from an irregular heart rhythm.The ECG from her initial visit is shown in Figure 1.What are possible diagnoses?Please turn the page to read the diagnosis.
Cardioprotection by Peroxidase Activity of Prostaglandin H Synthases in Ischemia/Reperfusion Injury
Authors: Guizhu Liu, Qian Liu, Naifu Wan, Xia Shen, Hui Cui, Cheng Dong, Xu Zhang, Huiyong Yin, Jian Wang, Colin D. Funk, Ying Yu
Journal: Circulation (2023)
Explaining declining hip fracture rates in Norway: a population-based modelling study
Authors: Helena Kames Kjeldgaard, Kristin Holvik, Bo Abrahamsen, Grethe S. Tell, Haakon E. Meyer, Martin O'Flaherty
Journal: The Lancet Regional Health - Europe (2023)

Although age-standardised hip fracture incidence has declined in many countries during recent decades, the number of fractures is forecast to increase as the population ages. Understanding the drivers behind this decline is essential to inform policy for targeted preventive measures. We aimed to quantify how much of this decline could be explained by temporal trends in major risk factors and osteoporosis treatment.We developed a new modelling approach, Hip-IMPACT, based on the validated IMPACT coronary heart disease models. The model applied sex- and age stratified hip fracture numbers and prevalence of pharmacologic treatments and risk/preventive factors in 1999 and 2019, and best available evidence for independent relative risks of hip fracture associated with each treatment and risk/preventive factor.Hip-IMPACT explained 91% (2500/2756) of the declining hip fracture rates during 1999-2019. Two-thirds of the total decline was attributed to changes in risk/preventive factors and one-fifth to osteoporosis medication. Increased prevalence of total hip replacements explained 474/2756 (17%), increased body mass index 698/2756 (25%), and increased physical activity 434/2756 (16%). Reduced smoking explained 293/2756 (11%), and reduced benzodiazepine use explained (366/2756) 13%. Increased uptake of alendronate, zoledronic acid, and denosumab explained 307/2756 (11%), 104/2756 (4%) and 161/2756 (6%), respectively. The explained decline was partially offset by increased prevalence of type 2 diabetes and users of glucocorticoids, z-drugs, and opioids.Two-thirds of the decline in hip fractures from 1999 to 2019 was attributed to reductions in major risk factors and approximately one-fifth to osteoporosis medication.The Research Council of Norway.
Allogeneic hematopoietic stem cell transplantation for NK/T-cell lymphoma: an international collaborative analysis
Authors: Philipp Berning, Norbert Schmitz, Maud Ngoya, Hervé Finel, Ariane Boumendil, Fengrong Wang, Xiao-Jun Huang, Olivier Hermine, Laure Philippe, Lucile Couronné, Arnaud Jaccard, Daihong Liu, Depei Wu, Hans Christian Reinhardt, Yves Chalandon, Eva Wagner-Drouet, Mi Kwon, Xi Zhang, Ben Carpenter, Ibrahim Yakoub-Agha, Gerald Wulf, Javier López-Jiménez, Jaime Sanz, Hélène Labussière-Wallet, Avichai Shimoni, Peter Dreger, Anna Sureda, Won Seog Kim, Bertram Glass
Journal: Leukemia (2023)

Abstract Natural killer/T-cell lymphomas (NKTCL) represent rare and aggressive lymphoid malignancies. Patients (pts) with relapsed/refractory disease after Asparaginase (ASPA)-based chemotherapy have a dismal prognosis. To better define the role of allogeneic hematopoietic stem cell transplantation (allo-HSCT), we conducted a retrospective analysis of data shared with the European Society for Blood and Marrow Transplantation (EBMT) and cooperating Asian centers. We identified 135 pts who received allo-HSCT between 2010 and 2020. Median age was 43.4 years at allo-HSCT, 68.1% were male. Ninety-seven pts (71.9 %) were European, 38 pts (28.1%) Asian. High Prognostic Index for NKTCL (PINK) scores were reported for 44.4%; 76.3% had &gt;1 treatment, 20.7% previous auto-HSCT, and 74.1% ASPA-containing regimens prior to allo-HSCT. Most (79.3%) pts were transplanted in CR/PR. With a median follow-up of 4.8 years, 3-year progression-free(PFS) and overall survival were 48.6% (95%-CI:39.5–57%) and 55.6% (95%-CI:46.5–63.8%). Non-relapse mortality at 1 year was 14.8% (95%-CI:9.3–21.5%) and 1-year relapse incidence 29.6% (95%-CI:21.9–37.6%). In multivariate analyses, shorter time interval (0–12 months) between diagnosis and allo-HSCT [HR = 2.12 (95%-CI:1.03–4.34); P = 0.04] and transplantation not in CR/PR [HR = 2.20 (95%-CI:0.98–4.95); P = 0.056] reduced PFS. Programmed cell death protein 1(PD-1/PD-L1) treatment before HSCT neither increased GVHD nor impacted survival. We demonstrate that allo-HSCT can achieve long-term survival in approximately half of pts allografted for NKTCL.
Correction: Triple MAPK inhibition salvaged a relapsed post-BCMA CAR-T cell therapy multiple myeloma patient with a BRAF V600E subclonal mutation
Authors: Muhammad Elnaggar, Sarita Agte, Paula Restrepo, Meghana Ram, David Melnekoff, Christos Adamopoulos, Mark M Stevens, Katerina Kappes, Violetta Leshchenko, Daniel Verina, Sundar Jagannath, Poulikos I Poulikakos, Samir Parekh, Alessandro Laganà
Journal: Journal of Hematology & Oncology (2023)
Correction: A novel lncRNA uc.134 represses hepatocellular carcinoma progression by inhibiting CUL4A-mediated ubiquitination of LATS1
Authors: Wen Ni, Yuqin Zhang, Zetao Zhan, Feng Ye, Yonghao Liang, Jing Huang, Keli Chen, Longhua Chen, Yi Ding
Journal: Journal of Hematology & Oncology (2023)
The Severity of Reduced Esophageal Distensibility Parallels Eosinophilic Esophagitis Disease Duration
Authors: Isis K. Araujo, Cristina Shehata, Ikuo Hirano, Nimala Gonsalves, Peter J. Kahrilas, Marie-Pier Tetreault, Jacob M. Schauer, Domenico Farina, Stephanie Peterson, Wenjun Kou, John E. Pandolfino, Dustin A. Carlson
Journal: Clinical Gastroenterology and Hepatology (2023)
Perspectives of Rare Disease Experts on Newborn Genome Sequencing
Authors: Nina B. Gold, Sophia M. Adelson, Nidhi Shah, Shardae Williams, Sarah L. Bick, Emilie S. Zoltick, Jessica I. Gold, Alanna Strong, Rebecca Ganetzky, Amy E. Roberts, Melissa Walker, Alexander M. Holtz, Vijay G. Sankaran, Ottavia Delmonte, Weizhen Tan, Ingrid A. Holm, Jay R. Thiagarajah, Junne Kamihara, Jason Comander, Emily Place, Janey Wiggs, Robert C. Green
Journal: JAMA Network Open (2023)

Importance Newborn genome sequencing (NBSeq) can detect infants at risk for treatable disorders currently undetected by conventional newborn screening. Despite broad stakeholder support for NBSeq, the perspectives of rare disease experts regarding which diseases should be screened have not been ascertained. Objective To query rare disease experts about their perspectives on NBSeq and which gene-disease pairs they consider appropriate to evaluate in apparently healthy newborns. Design, Setting, and Participants This survey study, designed between November 2, 2021, and February 11, 2022, assessed experts’ perspectives on 6 statements related to NBSeq. Experts were also asked to indicate whether they would recommend including each of 649 gene-disease pairs associated with potentially treatable conditions in NBSeq. The survey was administered between February 11 and September 23, 2022, to 386 experts, including all 144 directors of accredited medical and laboratory genetics training programs in the US. Exposures Expert perspectives on newborn screening using genome sequencing. Main Outcomes and Measures The proportion of experts indicating agreement or disagreement with each survey statement and those who selected inclusion of each gene-disease pair were tabulated. Exploratory analyses of responses by gender and age were conducted using t and χ 2 tests. Results Of 386 experts invited, 238 (61.7%) responded (mean [SD] age, 52.6 [12.8] years [range 27-93 years]; 126 [52.9%] women and 112 [47.1%] men). Among the experts who responded, 161 (87.9%) agreed that NBSeq for monogenic treatable disorders should be made available to all newborns; 107 (58.5%) agreed that NBSeq should include genes associated with treatable disorders, even if those conditions were low penetrance; 68 (37.2%) agreed that actionable adult-onset conditions should be sequenced in newborns to facilitate cascade testing in parents, and 51 (27.9%) agreed that NBSeq should include screening for conditions with no established therapies or management guidelines. The following 25 genes were recommended by 85% or more of the experts: OTC , G6PC , SLC37A4 , CYP11B1 , ARSB , F8 , F9 , SLC2A1 , CYP17A1 , RB1 , IDS , GUSB , DMD , GLUD1 , CYP11A1 , GALNS , CPS1 , PLPBP , ALDH7A1 , SLC26A3 , SLC25A15 , SMPD1 , GATM , SLC7A7 , and NAGS . Including these, 42 gene-disease pairs were endorsed by at least 80% of experts, and 432 genes were endorsed by at least 50% of experts. Conclusions and Relevance In this survey study, rare disease experts broadly supported NBSeq for treatable conditions and demonstrated substantial concordance regarding the inclusion of a specific subset of genes in NBSeq.
A deep learning algorithm to predict risk of pancreatic cancer from disease trajectories
Authors: Davide Placido, Bo Yuan, Jessica X Hjaltelin, Chunlei Zheng, Amalie D Haue, Piotr J Chmura, Chen Yuan, Jihye Kim, Renato Umeton, Gregory Antell, Alexander Chowdhury, Alexandra Franz, Lauren Brais, Elizabeth Andrews, Debora S Marks, Aviv Regev, Siamack Ayandeh, Mary T Brophy, Nhan V Do, Peter Kraft, Brian M Wolpin, Michael H Rosenthal, Nathanael R Fillmore, Søren Brunak, Chris Sander
Journal: Nature Medicine (2023)

Pancreatic cancer is an aggressive disease that typically presents late with poor outcomes, indicating a pronounced need for early detection. In this study, we applied artificial intelligence methods to clinical data from 6 million patients (24,000 pancreatic cancer cases) in Denmark (Danish National Patient Registry (DNPR)) and from 3 million patients (3,900 cases) in the United States (US Veterans Affairs (US-VA)). We trained machine learning models on the sequence of disease codes in clinical histories and tested prediction of cancer occurrence within incremental time windows (CancerRiskNet). For cancer occurrence within 36 months, the performance of the best DNPR model has area under the receiver operating characteristic (AUROC) curve = 0.88 and decreases to AUROC (3m) = 0.83 when disease events within 3 months before cancer diagnosis are excluded from training, with an estimated relative risk of 59 for 1,000 highest-risk patients older than age 50 years. Cross-application of the Danish model to US-VA data had lower performance (AUROC = 0.71), and retraining was needed to improve performance (AUROC = 0.78, AUROC (3m) = 0.76). These results improve the ability to design realistic surveillance programs for patients at elevated risk, potentially benefiting lifespan and quality of life by early detection of this aggressive cancer.
Inactivation of the antidiabetic drug acarbose by human intestinal microbial-mediated degradation
Authors: Jinzhong Tian, Chong Li, Zhixiang Dong, Yunpeng Yang, Jing Xing, Peijun Yu, Ying Xin, Fengmei Xu, Lianwei Wang, Yahui Mu, Xiangyang Guo, Qiang Sun, Guoping Zhao, Yang Gu, Guijun Qin, Weihong Jiang
Journal: Nature Metabolism (2023)
Changes in Physician Work Hours and Implications for Workforce Capacity, 2001 to 2021
Authors: Yi-Jui Chiu, Kuan-Yu Chiang, Nin-Chieh Hsu
Journal: JAMA Internal Medicine (2023)

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Genetic architecture of the inflammatory bowel diseases across East Asian and European ancestries
Authors: Zhanju Liu, Ruize Liu, Han Gao, Seulgi Jung, Xiang Gao, Ruicong Sun, Xiaoming Liu, Yongjae Kim, Ho-Su Lee, Yosuke Kawai, Masao Nagasaki, Junji Umeno, Katsushi Tokunaga, Yoshitaka Kinouchi, Atsushi Masamune, Wenzhao Shi, Chengguo Shen, Zhenglin Guo, Kai Yuan, Maria Abreu, Jean-Paul Achkar, Vibeke Andersen, Charles Bernstein, Steven R. Brant, Luis Bujanda, Siew Chien Ng, Lee A. Denson, Richard H. Duerr, Lynnette R. Ferguson, Denis Franchimont, Andre Franke, Richard Gearry, Hakon Hakonarson, Jonas Halfvarson, Caren Heller, Antonio Julià, Judith Kelsen, Hamed Khalili, Subramaniam Kugathasan, Juozas Kupcinskas, Anna Latiano, Edouard Louis, Reza Malekzadeh, Jacob L. McCauley, Christopher Moran, David Okou, Tim Orchard, Aarno Palotie, Miles Parkes, Joel Pekow, Uroš Potočnik, Graham Radford-Smith, John D. Rioux, Gerhard Rogler, Bruce Sands, Mark Silverberg, Harry Sokol, Séverine Vermeire, Rinse K. Weersma, Ramnik J. Xavier, Naizhong Hu, Qian Cao, Yufang Wang, Yinglei Miao, Hongjie Zhang, Xiaoping Lv, Xiang Gao, Hu Zhang, Jingling Su, Baisui Feng, Ye Zhao, Liangru Zhu, Yan Chen, Lanxiang Zhu, Chunxiao Chen, Yali Wang, Yingde Wang, Zhi Pang, Yingxuan Chen, Xiaolan Zhang, Hui Li, Qin Yu, Mei Ye, Sumin Zhang, Wen Tang, Mei Wang, Xiaocang Cao, Ruixin Zhu, Guangxi Zhou, Zhaolian Bian, Xiaofeng Guo, Xiaoli Wu, Jinchun Liu, Wei Xu, Yuqin Li, Qin Guo, Zhiguo Guo, Shu Zhu, Dalin Li, Jianjun Liu, Tian Ge, Judy Cho, Mark J. Daly, Dermot P. B. McGovern, Byong Duk Ye, Kyuyoung Song, Yoichi Kakuta, Mingsong Li, Hailiang Huang
Journal: Nature Genetics (2023)
Errors in Figure 2B
Authors: Frederick Green, Zeynep Guller, Suhail Aslam
Journal: JAMA Internal Medicine (2023)

Victoria T. Chu, MD, MPH; Noah G. Schwartz, MD; Marisa A. P. Donnelly, PhD; Meagan R. Chuey, PhD, RN; Raymond Soto, PhD; Anna R. Yousaf, MD; Emily N. Schmitt-Matzen, DVM, MPH; Sadia Sleweon, MPH; Jasmine Ruffin, MPH; Natalie Thornburg, PhD; Jennifer L. Harcourt, PhD; Azaibi Tamin, PhD; Gimin Kim, BS; Jennifer M. Folster, PhD; Laura J. Hughes, PhD; Suxiang Tong, PhD; Ginger Stringer, PhD, MPH; Bernadette A. Albanese, MD, MPH; Sarah E. Totten, DrPH; Meghan M. Hudziec, BS; Shannon R. Matzinger, PhD; Elizabeth A. Dietrich, PhD; Sarah W. Sheldon, MS; Sarah Stous, MPH; Eric C. McDonald, MD, MPH; Brett Austin, MA; Mark E. Beatty, MD, MPH; J. Erin Staples, MD, PhD; Marie E. Killerby, VetMB, MPH; Christopher H. Hsu, MD, PhD; Jacqueline E. Tate, PhD; Hannah L. Kirking, MD; Almea Matanock, MD, MS; COVID-19 Household Transmission Team; Winston E. Abara; Lorraine N. Alexander; Melissa M. Arons; Melanie J. Firestone; Monique A. Foster; Elizabeth Garza; Yessica Gomez; Sarah Anne J. Guagliardo; Dana L. Haberling; Ben W. Humrighouse; Stacey L. Konkle; Perrine Marcenac; Benjamin P. Monroe; Apophia Namageyo-Funa; Michelle O’Hegarty; Caroline Q. Pratt; Ian W. Pray; Byron F. Robinson; Horacio Ruiseñor-Escudero; Hannah E. Segaloff; Talya Shragai; Tarah S. Somers; Farrell A. Tobolowsky; Rachael L. Ticho Zacks; Raydel D. Anderson; Ayan K. Chakrabarti; Blake Cherney; Stephen P. LaVoie; Kaitlin F. Mitchell; Clint N. Morgan; Rebecca Rossetti; Robyn A. Stoddard; Jeni Vuong; Melissa J. Whaley; Joaudimir Castro Georgi; Rebecca J Chancey; Erica Figueroa; Jennifer Lehman; Kristine Lindell; Reed Magleby; Grace E. Marx; David W. McCormick; Paul Mead; Laird J. Ruth; Maggie Silver; Susanna N. Visser; Michelle A. Waltenburg; Karen L Boroughs; Aaron C. Brault; Anna Drexler; Janet McAllister; Jamie Pawloski; Janae Stovall; Maria I. Dionicio Bernabe; Anna Liza M. Manlutac; Graciela Zuniga-Groot; Seema Jain; Heather Baily; Alexis Burakoff; Christopher Grano; Lindsey Martin Webb; Evelyn Alvarez; Cassandra N Bundalian; Hannah Collins; Christopher Delmonico; Shaun Dunyak; Hannah M Fortune; Wyatt Giddmore; Kimberly Huynh-Templeman; Tia Ling; Hunter Olsen; Nicholas J Pysnack; Aleigha M Ricci; Tara M. Stitzlein; Jan Drobeniuc; Sabrina Weis-Torres; Lili Punkova; Tao Lily Jia; Peter Browning; Shanna Bolcen; Panagiotis Maniatis; So Hee Park; Kimberly M. Moss; Briana Zellner; Kristina Ortiz; Anna Uehara; Adam C. Retchless; Ying Tao; Yan Li; Anna Kelleher; Han Jia Justin NG; Jing Zhang; Brian Lynch; Peter W. Cook; Clinton R. Paden; Krista Queen; Magdalena Medrzycki; Shannon Rogers; Phili Wong; Shilpi Jain; Alexandra Tejada-Strop; John Michael Metz; Ebenezer David; Xiaoling Tang; Nhien T. Wynn; Rebekah Tiller; Senthilkumar K. Sakthivel; Srinivasan Velusamy; Brett Whitaker; Marla E. Petway